Clinical Study
β-Thalassemia Intermedia in Northern Iraq: A Single Center Experience
Table 1
Genotype, clinical, and hematological parameters in 74 patients with TI enrolled in the current study.
| | Number | Family | -Genotype | Xmn I status | -Genotype | Age
(yr) | Sex | Age at diagnosis (yr) | Age at first Tx. | Number of Tx. last yr. | Spleen cm BCM | Hb (g/dL) |
(%) | (%) |
| | 1 | F1 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 20 | F | 8 | 8 | 0 | 5 | 7.5 | 7.1 | 8.7 | | 2 | F1 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 21 | M | 10 | 10 | 0 | S | 8.0 | 8.4 | 11.1 | | 3 | F2 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 7 | M | 4 | NT | 0 | 3 | 7.0 | 6.9 | 8.7 | | 4 | F2 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 9 | F | 7 | NT | 0 | 3 | 7.6 | 6.1 | 14.9 | | 5 | F3 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 13 | M | 5 | 12 | 1 | 3 | 8.1 | 6.6 | 24.5 | | 6 | F4 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 19 | F | 4 | 4 | 2 | 3 | 7.5 | 5.8 | 17.6 | | 7 | F4 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 21 | M | 4 | 4 | 1 | 4 | 9.1 | 7.0 | 8.3 | | 8 | F5 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 38 | F | 24 | 24 | 4 | S | 9.3 | 8.0 | 10.0 | | 9 | F6 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 27 | F | 4 | 26 | 0 | 5 | 7.8 | 6.8 | 10.4 | | 10 | F6 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 33 | M | 5 | 5 | 0 | S | 8.2 | 8.5 | 7.7 | | 11 | F7 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 10 | F | 8 | NT | 0 | 1 | 7.3 | 7.2 | 9.8 | | 12 | F7 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 11 | F | 10 | NT | 0 | 1 | 7.3 | 6.4 | 12.3 | | 13 | F7 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 7 | F | 5 | NT | 0 | NP | 7.4 | 6.2 | 13.0 | | 14 | F8 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 5 | M | 1 | 1 | 2 | 3 | 7.8 | 4.5 | 17.3 | | 15 | F9 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 4 | M | 3 m | NT | 0 | 2 | 7.6 | 4.8 | 36.3 | | 16 | F10 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 3.0 | M | 3 | NT | 0 | 2 | 10.6 | 4.2 | 32.5 | | 17 | F11 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 31 | F | 6 | 6 | 12 | 3 | 9.1 | NA | NA | | 18 | F12 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 7 | F | 5 | NT | 0 | 7 | 7.2 | 7.3 | 29.4 | | 19 | F12 | IVS-I-6/IVS-I-6 | −/− | α α/α α | 15 | M | 5 | 5 | 0 | 7 | 6.8 | 7 | 26.9 | | 20 | F13 | IVS-I-128/IVS-I-128 | −/− | α α/α α | 24 | M | 11 | NT | 0 | 1 | 8.6 | 7 | 25 | | 21 | F13 | IVS-I-128/IVS-I-128 | −/− | α α/α α | 21 | F | 5 | 5 | 3 | 1 | 7.9 | 5 | 35 | | 22 | F14 |
−28/−28 | −/− | α α/α α | 33 | M | 15 | 15 | 2 | 6 | 7.6 | 7 | 18.5 | | 23 | F15 | IVS-1-6/−30 (T>A) | −/− | α α/α α | 33 | F | 11 | 11 | 0 | S | 8.3 | 5 | 20 | | 24 | F16 | IVS-I-6/IVS-II-1 | +/− | α α/α α | 8 | M | 7.5 | NT | 0 | 4 | 9.2 | 3.9 | 65.7 | | 25 | F17 | IVS-I-6/IVS-II-1 | +/− | α α/α α | 10 | M | 10 | NT | 0 | 2 | 8.3 | 3 | 62.2 | | 26 | F18 | IVS-I-6/Cd 82/83 | +/− | α α/α α | 6 | F | 9 m | 5 | 1 | 2 | 7.7 | 2.8 | 54.2 | | 27 | F18 | IVS-I-6/Cd 82/83 | +/− | α α/α α | 4 | M | 9 m | NT | 0 | 1 | 8.1 | 1.5 | 45.5 | | 28 | F18 | IVS-I-6/Cd 82/83 | +/− | α α/α α | 3 | F | 3 | NT | 0 | 2 | 8.3 | 2.0 | 60 | | 29 | F18 | IVS-I-6/Cd 82/83 | +/− | α α/α α | 13 | F | 6 | 6 | 10 | 1 | 8.9 | 2.8* | 45* | | 30 | F19 | IVS-I-6/Cd 39 | −/− | α α/α α | 5 | F | 6 m | 2 | 1 | 3 | 8.0 | 3.5 | 56.0 | | 31 | F20 | IVS-I-6/Cd 5 | −/− | α α/α α | 46 | F | 34 | 40 | 0 | S | 7.8 | 5.1 | 87.2 | | 32 | F20 | IVS-I-6/Cd 5 | −/− | α α/α α | 49 | F | 37 | 42 | 0 | S | 7.2 | 8 | 80.1 | | 33 | F21 | IVS-1-6/Cd 8 | +/− | α α/α α | 25 | F | 24 | 24 | 8 | 7 | 8.0 | 3.3* | 58* | | 34 | F22 | IVS-I-6/Cd 8 | +/− | α α/α α | 2.5 | F | 2.5 | 2.5 | 1 | 2 | 6.9 | 5.5 | 46.6 | | 35 | F22 | IVS-I-6/Cd 8 | +/− | α α/α α | 13 | F | 5 | 5 | 12 | S | 9.6 | NA | NA | | 36 | F22 | IVS-I-6/Cd 8 | +/− | α α/α α | 5 | F | 4 | 4 | 2 | 4 | 7.2 | 6.1 | 48 | | 37 | F23 | IVS-1-6/IVS-I-1 | −/− | α α/α α | 29 | F | 6 | 6 | 12 | S | 10.6 | NA | NA | | 38 | F24 | IVS-I-130/Poly A | −/− | α α/α α | 32 | M | 8 | 8 | 0 | 7 | 6.5 | 3 | 46 | | 39 | F25 |
Cd 5/Poly A | +/− | α α/α α | 16 | M | 3 | 3 | 3 | 7 | 7 | 4.2 | 35.4 | | 40 | F25 | Cd 5/Poly A | +/− | α α/α α | 18 | M | 3 | 3 | 0 | S | 6.7 | 5.7 | 37.9 | | 41 | F25 | Cd 5/Poly A | +/− | α α/α α | 7 | F | 3 | 3 | 4 | 4 | 6.8 | 4.0 | 33.9 | | 42 | F25 | Cd 5/Poly A | +/− | α α/α α | 22 | F | 2 | 2 | 0 | S | 7.9 | 6.4 | 35.4 | | 43 | F25 | Cd 5/Poly A | +/− | α α/α α | 8 | F | 6 | NT | 0 | 2 | 7.1 | 3.8 | 49.1 | | 44 | F26 | Cd 5/Poly A | +/− | α α/α α | 23 | F | 5 | 5 | 0 | 4 | 8.6 | 6.1 | 41.6 | | 45 | F26 | Cd 5/Poly A | +/− | α α/α α | 36 | F | 17 | 17 | 0 | 3 | 7.3 | 6.6 | 37.1 | | 46 | F26 | Cd 5/Poly A | +/− | α α/α α | 19 | M | 7 | 7 | 0 | 3 | 7.4 | 6.3 | 36.8 | | 47 | F27 | IVS-II-1/IVS-II-1 | +/+ | α α/α α | 19 | M | 1 | NT | 0 | S | 9.5 | 1.5 | 98.5 | | 48 | F27 | IVS-II-1/IVS-II-1 | +/+ | α α/α α | 8 | M | 1 | NT | 0 | S | 9.3 | 3 | 96 | | 49 | F28 | IVS-II-1/IVS-II-1 | +/+ | α α/α α | 14 | M | 10 | NT | 0 | 4 | 11 | 3.5 | 96.5 | | 50 | F28 | IVS-II-1/IVS-II-1 | +/+ | α α/α α | 17 | F | 3 | 3 | 1 | 5 | 11.6 | 4.0 | 96 | | 51 | F29 | IVS-II-1/IVS-II-1 | +/+ | α α/α α | 7 | F | 4 | 4 | 0 | 6 | 7.5 | 1.8 | 96.3 | | 52 | F30 | IVS-II-1/IVS-II-1 | +/+ | α α/α α | 18 | M | 16 | NT | 0 | 2 | 10 | 2.6 | 97 | | 53 | F31 | Cd 82/83/Cd 82/83 | +/+ | α α/α α | 29 | M | 9 | 9 | 4 | S | 9.9 | 2.7 | 91.7 | | 54 | F32 | Cd 82/83/Cd 82/83 | +/+ | α α/α α | 26 | M | 5 | 5 | 0 | 10 | 7.6 | 2.5 | 97.5 | | 55 | F33 | Cd 82/83/Cd 82/83 | +/+ | α α/α α | 25 | M | 19 | 19 | 0 | 1 | 11.0 | 1.6 | 98.3 | | 56 | F34 | Cd 82/83/Cd 82/83 | +/+ | α α/α α | 8 | M | 2.5 | 2.5 | 0 | 4 | 7.3 | 3.8 | 95 | | 57 | F35 | Cd 8/Cd 8 | +/+ | α α/α α | 19 | F | 7 | 7 | 3 | S | 9.4 | 2.0 | 94 | | 58 | F36 | IVS-II-1/Cd 82/83 | +/+ | α α/α α | 9 | M | 1 | 1 | 5 | 3 | 9.5 | 4.7* | 95.0* | | 59 | F37 | IVS-II-1/Cd 8 | +/+ | α α/α α | 7 | M | 7 | NT | 0 | 3 | 7.5 | 2 | 98 | | 60 | F38 | Cd 8/IVS-II-1 | +/− | α α/α α | 12 | M | 5 | 5 | 12 | 2 | 6.9 | 2.5* | 97.5* | | 61 | F38 | Cd 8/IVS-II-1 | +/− | α α/α α | 5 | F | 9 m | NT | 0 | 1 | 8.7 | 2.0 | 98 | | 62 | F39 | IVS-II-1/Cd 44 | +/− | α α/α α | 5 | F | 2 | 2.5 | 2 | 2 | 8.1 | 1.8 | 98.2 | | 63 | F40 | IVS-II-1/Cd 44 | +/− | /α α | 18 | M | 2 | NT | 0 | 2 | 9.7 | 2.3 | 97.7 | | 64 | F41 | IVS-II-1/IVS-I-1 | +/− | α α/α α | 3 | M | 8 m | NT | 0 | NP | 7.4 | 3 | 96.8 | | 65 | F42 | IVS-II-1/IVS-I-1 | +/− | /α α | 5 | M | 4 | 4 | 10 | 1 | 6.7 | 2* | 98* | | 66 | F43 | Cd 8/IVS-I-110 | +/− | /α α | 21 | M | 1 | 1 | 0 | S | 10 | 5 | 87 | | 67 | F44 | IVS-II-1/IVS1-110 | +/− | α α/α α | 12 | M | 5 | 5 | 2 | 7 | 8.3 | 2.9 | 55 | | 68 | F45 | Cd 8/IVS-I-1 | +/− | α α/α α | 4 | M | 2 | 4 | 1 | 1 | 7.7 | 1.6 | 98 | | 69 | F46 | IVS-II-1/IVS-II-1 | +/− | /α α | 18 | F | 6 | 6 | 0 | 3 | 10 | 2.7 | 97.3 | | 70 | F47 | IVS-I-130/Cd 5 | −/− | α α/α α | 16 | F | 2 | 2 | 0 | S | 9.6 | 2.5 | 97.4 | | 71 | F48 | IVS-II-1/IVS-1-130 | +/− | α α/α α | 20 | F | 3 | NT | 0 | S | 8.3 | 5 | 95 | | 72 | F49 | Cd 5/Wild | −/− | α α/α α | 8 | F | 2 | NT | 0 | 3 | 6.9 | 5.8 | 5.7 | | 73 | F50 | Cd 5/Wild | −/− | α α/α αα | 17 | F | 13 | 13 | 4 | 5 | 6.5 | 4.5 | 3.3 | | 74 | F51 | Cd 39/Wild | −/− | α α/α α | 30 | M | 19 | 19 | 1 | 1 | 9 | 4.9 | 8.8 |
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NA: Not Available; NP: Not Palpable; *Parameter at diagnosis; S: Splenectomy; NT: never transfused; M: Male; F: Female; Poly A: AATAAA>AATAAG mutation; Tx: transfusion.
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